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What is cancer?
The body is made up of trillions of living cells. Normal body cells grow, divide into new
cells, and die in an orderly way. During the early years of a person's life, normal cells divide
faster to allow the person to grow. After the person becomes an adult, most cells divide only
to replace worn-out or dying cells or to repair injuries.
Cancer begins when cells in a part of the body start to grow out of control. There are many
kinds of cancer, but they all start because of out-of-control growth of abnormal cells.
Cancer cell growth is different from normal cell growth. Instead of dying, cancer cells
continue to grow and form new, abnormal cells. Cancer cells can also invade (grow into)
other tissues, something that normal cells cannot do. Growing out of control and invading
other tissues are what makes a cell a cancer cell.
Cells become cancer cells because of damage to DNA. DNA is in every cell and directs all its
actions. In a normal cell, when DNA gets damaged the cell either repairs the damage or the
cell dies. In cancer cells, the damaged DNA is not repaired, but the cell doesnt die like it
should. Instead, this cell goes on making new cells that the body does not need. These new
cells will all have the same damaged DNA as the first cell does.
People can inherit damaged DNA, but most DNA damage is caused by mistakes that happen
while the normal cell is reproducing or by something in our environment. Sometimes the
cause of the DNA damage is something obvious, like cigarette smoking. But often no clear
cause is found.
In most cases the cancer cells form a tumor. Some cancers, like leukemia, rarely form
tumors. Instead, these cancer cells involve the blood and blood-forming organs and circulate
through other tissues where they grow.
Cancer cells often travel to other parts of the body, where they begin to grow and form new
tumors that replace normal tissue. This process is called metastasis. It happens when the
cancer cells get into the bloodstream or lymph vessels of our body.
No matter where a cancer may spread, it is always named for the place where it started. For
example, breast cancer that has spread to the liver is still breast cancer, not liver cancer.
Likewise, prostate cancer that has spread to the bone is metastatic prostate cancer, not bone
cancer.
Different types of cancer can behave very differently. For example, lung cancer and breast
cancer are very different diseases. They grow at different rates and respond to different
treatments. That is why people with cancer need treatment that is aimed at their particular
kind of cancer.
Not all tumors are cancerous. Tumors that arent cancer are called benign. Benign tumors can
cause problems they can grow very large and press on healthy organs and tissues. But
they cannot grow into (invade) other tissues. Because they cant invade, they also cant
spread to other parts of the body (metastasize). These tumors are almost never lifethreatening.
Hibernomas, like lipomas, are also benign fat tissue tumors. They are much less common
than lipomas.
Scientists have found a few risk factors that make a person more likely to develop soft tissue
sarcomas.
Radiation exposure
Patients might develop sarcomas from radiation given to treat other cancers, like breast
cancer or lymphoma. The sarcoma often starts in the area of the body that had been treated
with radiation. The average time between radiation exposure and diagnosis of a sarcoma is
about 10 years. Radiation exposure accounts for less than 5% of sarcomas.
Radiation therapy techniques have improved steadily over several decades. Treatments now
target the cancers more precisely, and more is known about selecting radiation doses. These
advances are expected to reduce the number of cancers caused by radiation therapy. But
because these cancers take so long to develop, the results of these changes may not be seen
for a long time. Still, radiation therapy is used only when its benefits (improved survival rate
and relief of symptoms) outweigh the risk of cancer and other complications. For more
information, see our document Second Cancers Caused by Cancer Treatment.
Genetic syndromes
Certain inherited conditions increase a person's risk of developing soft tissue sarcomas.
Neurofibromatosis
Neurofibromatosis is a disease that usually runs in families and is characterized by many
neurofibromas (benign tumors that form in nerves under the skin and in other parts of the
body). It is also known as von Recklinghausen disease. It is caused by a defect (mutation) in
a gene called NF1. About 5% of people with neurofibromatosis will develop a malignant
peripheral nerve sheath tumor in a neurofibroma.
Gardner syndrome
Gardner syndrome is a disease caused by defects in the APC gene. People with this syndrome
get many polyps in the colon (and intestines) and have a high risk of getting colon cancer. It
also causes musculoaponeurotic fibromatosis (also called desmoid tumors). Some experts
consider desmoid tumors a slow-growing (low-grade) type of fibrosarcoma.
Li-Fraumeni syndrome
Li-Fraumeni syndrome is caused by inherited defects in the TP53 gene. People affected by
this syndrome have a high risk of cancer, such as breast cancer, brain tumors, and sarcomas.
People with this syndrome are sensitive to the cancer-causing effects of radiation if their
cancer is treated with radiation, they have a very high chance of developing a new cancer in
the part of the body that received the radiation.
Retinoblastoma
Retinoblastoma is an eye cancer in children that can be caused by defects in the RB1 gene.
Children with one of these gene defects also have a higher risk of developing bone or soft
tissue sarcomas, especially if treated for cancer with radiation.
Werner syndrome
Werner syndrome is caused by defects in the RECQL2 gene. Children with this syndrome
have problems like those seen in the elderly. These include cataracts, skin changes, and
clogged heart arteries (arteriosclerosis) which can lead to heart attacks. They also have an
increased risk of cancer, including soft tissue sarcomas.
Gorlin syndrome
Gorlin syndrome, also called nevoid basal cell carcinoma syndrome, is caused by defects in
the PTCH1 gene. People with this syndrome have a high risk of developing many basal cell
skin cancers. They also have an increased risk of getting fibrosarcoma and
rhabdomyosarcoma.
Tuberous sclerosis
Tuberous sclerosis can be caused by a defect in the TSC1 gene. It can also be caused by a
defect in another gene: TSC2. People with this syndrome often have seizures and learning
problems. They get benign tumors in many different organs. They also get kidney problems,
often along with a kidney tumor called angiomyolipoma. People with tuberous sclerosis have
an increased risk of getting rhabdomyosarcoma.
Chemicals
Exposure to vinyl chloride (a chemical used in making plastics) is a risk factor for
developing sarcomas of the liver, but it has not been proven to cause soft tissue sarcomas.
Arsenic has also been linked to a type of liver sarcoma but not soft tissue sarcoma. Exposure
to dioxin and to herbicides that contain phenoxyacetic acid at high doses (such as might
occur to people who work on farms) may also be risk factors, but this is not known for
certain. There is no evidence that herbicides (weed killers) or insecticides, at levels
encountered by the general public, cause sarcomas.
Injury
An injury is not a risk factor for developing sarcomas. But this issue has caused some
confusion in the past. One reason is that injury may produce a swelling that resembles a
tumor but is not a true tumor. Also, when you are injured, the pain may draw your attention
to the injured area. The area may be examined closely, and x-rays or other imaging studies
may be obtained. This can make it more likely that any sarcoma that is present will be
discovered, even though it may have been present for some time.
characterized many of these DNA changes in the past few years. To learn more about
inherited genetic conditions, see Family Cancer Syndromes.
Some inherited conditions that increase a person's risk of developing soft tissue sarcoma
were noted in the section on risk factors. They are caused by defects (mutations) in genes that
can be inherited from a parent. These gene defects can be found through genetic testing. For
more on this topic, see Genetic Testing: What You Need to Know.
DNA mutations in soft tissue sarcoma are common. They are usually acquired during life
rather than having been inherited before birth. Acquired mutations may result from exposure
to radiation or cancer-causing chemicals. In most sarcomas, they occur for no apparent
reason.
Researchers still do not know why most soft tissue sarcomas develop in people who have no
apparent risk factors.
Imaging tests
Some tests, such as a computed tomography (CT) scan or a magnetic resonance imaging
(MRI) scan, are often done to look for the cause of symptoms and to find a tumor (such as a
sarcoma). Other tests may be done after a sarcoma is diagnosed to look for cancer spread.
Plain x-ray
A regular x-ray of the area with the lump may be the first test ordered. A plain chest x-ray
may be done after diagnosis to look for spread of sarcoma to the lungs.
Ultrasound
Ultrasound uses sound waves and their echoes to produce pictures of parts of the body. A
small instrument called a transducer emits sound waves and picks up the echoes as they
bounce off the organs. The sound wave echoes are converted by a computer into an image
that is displayed on a computer screen.
This is a very easy procedure to have. It uses no radiation, which is why it is often used to
look at developing fetuses. For most ultrasounds, you simply lie on a table while a technician
moves the transducer over the part of your body being examined. Usually, the skin is first
lubricated with gel. This test may be done before a biopsy to see if the lump is a cyst,
meaning it contains fluid and is likely benign, or if it is solid and more likely a tumor. This
test is often not needed if a CT or MRI was done.
different x-rays because it scans your whole body. Often the PET scan is used with a CT
scan. This helps decide if abnormalities seen on the CT scan are cancer or something else.
PET is not often used for sarcoma, but it can be helpful in certain cases.
Biopsy
A biopsy is a procedure that removes a sample of tissue from a tumor to see if it is a type of
cancer. The piece of tissue is looked at under a microscope and, some other tests may be
done on the sample as well. A physical exam or imaging test may suggest that a tumor is a
sarcoma, but a biopsy is the only way to be certain that it is a sarcoma and not another type
of cancer or a benign disease.
Several types of biopsies are used to diagnose sarcomas. Doctors with experience in these
tumors will choose one, based on the size and location of the tumor. Most experts prefer a
fine needle aspiration or a core needle biopsy as the first step.
Surgical biopsy
In a surgical biopsy, the entire tumor or a piece of the tumor is removed during an operation.
There are 2 types of surgical biopsies, excisional and incisional. In an excisional biopsy, the
surgeon removes the entire tumor. In an incisional biopsy, only a piece of a large tumor is
removed. An incisional biopsy almost always removes enough tissue to diagnose the exact
type and grade of sarcoma. If the tumor is near the skin surface, this is a simple operation that
can be done with local or regional anesthesia (numbing medication given near the mass or
into a nerve). But if the tumor is deep inside the body, general anesthesia is used (the patient
is asleep).
If a tumor is rather small, near the surface of the body, and not located near critical tissues
(such as important nerves or large blood vessels), the doctor may choose to remove the entire
mass and a margin of normal tissue in an excisional biopsy. This surgery combines the
biopsy and the treatment into one operation, so it should only be done by a surgeon with
experience in treating sarcomas.
If the tumor is large, then an incisional biopsy is needed. Only a surgeon experienced in
sarcoma treatment should perform this procedure.
You might want to ask about your surgeons experience with this procedure. Proper biopsy
technique is a very important part of successfully treating soft tissue sarcomas. An improper
biopsy can lead to tumor spread and problems removing the tumor later on. An incisional
biopsy in the wrong place or an excision without wide enough margins can make it harder to
completely remove a sarcoma later on. To prevent these problems, these 2 types of biopsies
should only be done by a surgeon experienced in treating sarcomas. It is best that an
incisional biopsy be done by the same surgeon who will later remove the entire tumor (if a
sarcoma is found).
A staging system is a standard way for the cancer care team to summarize the extent of a
cancer's spread. The system often used to stage sarcomas is the TNM system of American
Joint Committee on Cancer.
T stands for the size of the tumor.
N stands for spread to lymph nodes (small bean-shaped collections of immune system
cells found throughout the body that help fight infections and cancers).
M is for metastasis (spread to distant organs).
In soft tissue sarcomas, an additional factor, called grade (G), is also part of the tumors
stage. The grade is based on how the sarcoma cells look under the microscope.
Grade (G)
The grade is a sign of how likely it is the cancer will spread. Previously, the grade of a
sarcoma was only based on how normal the cells looked under the microscope (called
differentiation). This was not very helpful, and under a new system (known as the French or
FNCLCC system), grade is based on 3 factors:
Differentiation cancer cells are given a score of 1 to 3, with 1 being assigned when
they look similar to normal cells and 3 being used when the cancer cells look very
abnormal
Mitotic count how many cancer cells are seen dividing under the microscope; given a
score from 1 to 3 (a lower score means fewer cells were seen dividing)
Tumor necrosis how much of the tumor is made up of dying tissue; given a score from
0 to 2 (a lower score means there was less dying tissue present).
The scores for each factor are added to determine the grade for the cancer. Higher-grade
cancers tend to grow and spread faster than lower-grade cancers.
GX: the grade cannot be assessed (because of incomplete information).
Grade 1 (G1): Total score of 2 or 3
Grade 2 (G2): Total score of 4 or 5
Grade 3 (G3): Total score of 6 or higher
Tumor (T)
T1: The sarcoma is 5 cm (2 inches) or less across
T1a: The tumor is superficial near the surface of the body.
Metastasis (M)
M0: No distant metastases (spread) of sarcoma are found.
M1: The sarcoma has spread to distant organs or tissues (such as the lungs).
Stage IA
T1, N0, M0, G1 or GX: The tumor is not larger than 5 cm (2 inches) across (T1). It has not
spread to lymph nodes (N0) or more distant sites (M0). The cancer is grade 1 (or the grade
cannot be assessed).
Stage IB
T2, N0, M0, G1 or GX: The tumor is larger than 5 cm (2 inches) across (T2). It has not
spread to lymph nodes (N0) or more distant sites (M0). The cancer is grade 1 (or the grade
cannot be assessed).
Stage IIA
T1, N0, M0, G2 or G3: The tumor is not larger than 5 cm (2 inches) across (T1). It has not
spread to lymph nodes (N0) or more distant sites (M0). The cancer is grade 2 or 3.
Stage IIB
T2, N0, M0, G2: The tumor is larger than 5 cm (2 inches) across (T2). It has not spread to
lymph nodes (N0) or more distant sites (M0). The cancer is grade 2.
Stage III:
Either
T2, N0, M0, G3: It is larger than 5 cm (2 inches) across (T2). It has not spread to lymph
nodes (N0) or more distant sites (M0). The cancer is grade 3.
OR
Any T, N1, M0, any G: The cancer can be any size (any T) and any grade. It has spread to
nearby lymph nodes (N1). It has not spread to distant sites (M0).
Stage IV
Any T, Any N, M1, any G: The cancer can be any size (any T) and grade (any G). It has
spread to lymph nodes near the tumor (N1) and/or to distant sites (M1).
worse outcomes than younger people. Your doctor can tell you how the numbers below may
apply to you, as he or she is familiar with your particular situation.
The rates below are based on the stage of the cancer at the time of diagnosis. When looking
at survival rates, its important to understand that the stage of a cancer does not change over
time, even if the cancer progresses. A cancer that comes back or spreads is still referred to by
the stage it was given when it was first found and diagnosed, but more information is added
to explain the current extent of the cancer. (And the treatment plan is adjusted based on the
change in cancer status.)
The overall relative 5-year survival rate of people with soft tissue sarcomas is around 50%
according to statistics from the National Cancer Institute (NCI). These statistics include
people with Kaposi sarcoma, which has a poorer outlook than many sarcomas. The NCI
doesnt use the AJCC staging system. Instead, they group sarcomas only by whether they are
still confined to the primary site (called localized) have spread to nearby lymph nodes or
tissues (called regional); or have spread (metastasized) to sites away from the main tumor
(called distant). The 5-year survival rates for soft tissue sarcomas have not changed much for
many years. The corresponding 5-year relative survival rates were:
83% for localized sarcomas (56% of soft tissue sarcomas were localized when they were
diagnosed)
54% for regional stage sarcomas; (19% were in this stage)
16% for sarcomas with distant spread (16% were in this stage)
The 10-year relative survival rate is only slightly worse for these stages, meaning that most
people who survive 5 years are probably cured.
For sarcomas of the arms and legs, Memorial Sloan-Kettering Cancer Center has survival
rates broken down by AJCC stage (these are for observed, not relative survival):
Stage
5-year observed
survival rate
90%
II
81%
III
56%
IV
Not available
Survival is worse when the sarcoma has developed somewhere other than the arms or legs.
For example, the 5-year survival for retroperitoneal sarcomas is around 40% to 60%.
Radiation
Chemotherapy
Targeted therapy
It is important to discuss all of your treatment options, including their goals and possible side
effects, with your doctors to help make the decision that best fits your needs. Its also very
important to ask questions if there is anything youre not sure about. You can find some good
questions to ask in the section, What should you ask your doctor about soft tissue
sarcomas? It also is often a good idea to seek a second opinion. A second opinion can
provide more information and help you feel more confident about the chosen treatment plan.
Some insurance companies require a second opinion before they will agree to pay for
treatments.
Sometimes chemotherapy (chemo), radiation, or both is given before surgery. This, called
neoadjuvant treatment, can shrink the tumor and allow it to be removed completely. Chemo
or radiation can also be given before surgery to treat high-grade sarcomas when there is a
great risk of the cancer spreading.
Most of the time, surgery cannot cure a sarcoma once it has spread. But if it has only spread
to the lung, the metastatic tumor can sometimes be removed. This can cure many patients, or
at least lead to long-term survival.
You can read more about surgery for cancer in our document, Understanding Cancer
Surgery: A Guide for Patients and Families.
only one part of radiation treatment, and the patient receives some other type of radiation
after surgery.
Brachytherapy: Brachytherapy (sometimes called internal radiation therapy) is a treatment
that places small pellets (or seeds) of radioactive material in or near the cancer. For soft
tissue sarcoma, these pellets are put into catheters (very thin tubes) that have been placed
during surgery. In high-dose rate (HDR) brachytherapy, the pellets give off a lot of radiation
in a short time, and so stay in place for only minutes at a time. In low-dose rate (LDR)
brachytherapy, the pellets may stay in place for days at a time, and then removed.
Brachytherapy may be the only form of radiation therapy used or it can be combined with
external beam radiation.
Some chemo side effects can last a long time or even be permanent. For example,
doxorubicin can weaken the heart if too much is given. If you are to be treated with this drug,
your doctor might check your heart function with special studies before starting this drug.
The doctor will also watch the dose of doxorubicin closely during therapy.
Some chemo drugs cause nerve damage (called neuropathy), leading to numbness, tingling,
or even pain in the hands and feet. To learn more about this, see our document Peripheral
Neuropathy After Chemotherapy.
Chemotherapy may also permanently damage ovaries or testicles, which can lead to
infertility (not being able to have children). This is discussed in more detail in our documents
Fertility and Women With Cancer and Fertility and Men With Cancer.
If youd like more information on a drug used in your treatment or a specific drug mentioned
in this section, see our Guide to Cancer Drugs, ask your health care team, or call us with the
names of the medicines youre taking.
Pazopanib (Votrient)
At this time, pazopanib is the only targeted therapy drug approved to treat soft tissue
sarcoma. It blocks several cellular enzymes called tyrosine kinases that are important for cell
growth and survival. In a study of patients with advanced soft tissue sarcomas that had been
treated with chemotherapy, pazopanib stopped the cancers from growing for an average of
about 3 months longer than the patients given a sugar pill. So far, though, this drug hasnt
been shown to help patients live longer. This drug is taken in pill form, once a day.
Common side effects include high blood pressure, nausea, diarrhea, headaches, low blood
cell counts, and liver problems. In some patients this drug causes abnormal results on liver
function tests, but it also rarely leads to severe liver damage that can be life threatening.
Bleeding, clotting, and wound healing problems can occur, as well. This drug also rarely
causes a problem with the heart rhythm or even a heart attack. If you are taking pazopanib,
your doctor will monitor your heart with EKGs as well as check your blood tests to check for
liver or other problems.
Imatinib (Gleevec)
Imatinib is a tyrosine kinase inhibitor drug approved to treat gastrointestinal stromal tumors
and some kinds of leukemia. It also can be helpful in treating desmoid tumors that cant be
removed with surgery. Although it rarely causes tumors to shrink, it often causes them to
stop growing for a time, which can be very helpful.
Side effects can include mild stomach upset, diarrhea, muscle pain, and skin rashes. The
stomach upset is lessened if the drug is taken with food. Imatinib can also make people retain
fluid. Often this causes some swelling in the face (around the eyes) or in the ankles. Rarely
the drug causes more severe problems, such as fluid build up in the lungs or abdomen or
causing problems with heart function.
If youd like more information on a drug used in your treatment or a specific drug mentioned
in this section, ask your health care team, see our Guide to Cancer Drugs, or call us with the
names of the medicines youre taking.
Clinical trials are one way to get state-of-the art cancer treatment. In some cases they may be
the only way to get access to newer treatments. They are also the only way for doctors to
learn better methods to treat cancer. Still, they are not right for everyone.
You can get a lot more information on clinical trials in our document called Clinical Trials:
What You Need to Know. You can read it on our website or call our toll-free number (1-800227-2345) and have it sent to you.
longer be working. But the truth is that most of these alternative methods have not been
tested and proven to work in treating cancer.
As you consider your options, here are 3 important steps you can take:
Look for "red flags" that suggest fraud. Does the method promise to cure all or most
cancers? Are you told not to have regular medical treatments? Is the treatment a "secret"
that requires you to visit certain providers or travel to another country?
Talk to your doctor or nurse about any method you are thinking about using.
Contact us at 1-800-227-2345 to learn more about complementary and alternative
methods in general and to find out about the specific methods that interest you. You can
also read about them on the Complementary and Alternative Medicine page of our
website.
Desmoid tumors
Desmoid tumors are often not considered true cancers because they do not spread to distant
sites. The most common treatment for these tumors is surgery. If the entire tumor is removed
and the margins are clear, no other treatment is needed. These tumors can also be treated with
radiation (instead of surgery).
For tumors that are large or have come back after treatment, drug therapy may be helpful.
The drug sulindac, normally used to treat arthritis, can stop tumor growth or even cause the
tumor to shrink. It can take months for the drug to work, but its effect can last for years.
Drugs that block estrogen (tamoxifen and toremifene) have also been helpful in some
patients. Some desmoid tumors have responded to treatment with chemotherapy (chemo)
using the drug doxorubicin (Adriamycin), which may be used alone or with other drugs. The
combination of methotrexate and vinblastine has also been helpful. Interferon, an immuneboosting drug, has also been used with some success. Another option is the targeted drug
imatinib (Gleevec).
or the patient's health in general makes surgery impossible. There is evidence that chemo
after surgery may benefit some people with stage II and III sarcomas.
Recurrent sarcoma
Cancer is called recurrent when it come backs after treatment. Recurrence can be local (in or
near the same place it started) or distant (spread to other organs or tissues such as the lungs or
brain). If the sarcoma comes back in the same area where it started, it may be treated with
surgery. Radiation therapy is another option, especially if radiation wasnt part of the
treatment of the original tumor. If external beam radiation was used before, brachytherapy
may still be an option.
If the sarcoma returns at a distant site, chemo may be given. If the sarcoma has spread only to
the lungs, it may be possible to remove all the areas of spread with surgery. Radiation is used
to treat sarcomas that spread to the brain, as well as any recurrences that cause symptoms
such as pain.
The NCI provides treatment information via telephone (1-800-4-CANCER) and its website
(www.cancer.gov). Information for patients as well as more detailed information for cancer
care professionals is also available on www.cancer.gov.
No cancer treatment can be done without consent. People who are capable of making their
own decisions can decide whether they are willing to take any medical treatment, including
any recommended cancer treatments. Your doctor should discuss treatment options with you,
and explain what is likely to happen if you accept or refuse these treatments. For more, you
can read our document Informed Consent.
Follow-up care
When treatment ends, your doctors will still want to watch you closely. It is very important to
go to all of your follow-up appointments. During these visits, your doctors will ask questions
about any problems you have and might do exams and lab tests or x-rays and scans to look
for signs of cancer or treatment side effects. Almost any cancer treatment can have side
effects. Some may last for a few weeks to months, but others can last the rest of your life.
This is the time for you to talk to your cancer care team about any changes or problems you
notice and any questions or concerns you have.
It is important to keep health insurance. Tests and doctor visits cost a lot, and even though no
one wants to think about their cancer coming back, this could happen.
Should your cancer come back, our document, When Your Cancer Comes Back: Cancer
Recurrence can give you information to help you manage and cope with this phase of your
treatment.
Eating better
Eating right can be hard for anyone, but it can get even tougher during and after cancer
treatment. Treatment may change your sense of taste. Nausea can be a problem. You may not
feel like eating and lose weight when you don't want to. Or you may have gained weight that
you can't seem to lose. All of these things can be very frustrating.
If treatment caused weight changes or eating or taste problems, do the best you can and keep
in mind that these problems usually get better over time. You may find it helps to eat small
portions every 2 to 3 hours until you feel better. You might also want to ask your cancer team
about seeing a dietitian, an expert in nutrition who can give you ideas on how to deal with
these treatment side effects.
One of the best things you can do after cancer treatment is put healthy eating habits into
place. You may be surprised at the long-term benefits of some simple changes, like
increasing the variety of healthy foods you eat. Getting to and staying at a healthy weight,
eating a healthy diet, and limiting your alcohol intake may lower your risk for a number of
types of cancer, as well as having many other health benefits. For more information, see our
document, Nutrition and Physical Activity During and After Cancer Treatment: Answers to
Common Questions.
Keep in mind exercise can improve your physical and emotional health.
It improves your cardiovascular (heart and circulation) fitness.
Along with a good diet, it will help you get to and stay at a healthy weight.
It makes your muscles stronger.
It reduces fatigue and helps you have more energy.
It can help lower anxiety and depression.
It can make you feel happier.
It helps you feel better about yourself.
And long term, we know that getting regular physical activity plays a role in helping to lower
the risk of some cancers, as well as having other health benefits.
issues common to people with cancer in our booklet, Distress in People With Cancer, which
can be ordered from our toll-free number at no cost to you.
given at home. Your cancer may be causing problems that need to be managed, and hospice
focuses on your comfort. You should know that while getting hospice care often means the
end of treatments such as chemo and radiation, it doesn't mean you can't have treatment for
the problems caused by your cancer or other health conditions. In hospice the focus of your
care is on living life as fully as possible and feeling as well as you can at this difficult time.
You can learn more in our document called Hospice Care.
Staying hopeful is important, too. Your hope for a cure may not be as bright, but there is still
hope for good times with family and friends times that are filled with happiness and
meaning. Pausing at this time in your cancer treatment gives you a chance to refocus on the
most important things in your life. Now is the time to do some things you've always wanted
to do and to stop doing the things you no longer want to do. Though the cancer may be
beyond your control, there are still choices you can make.
Basic research
Scientists have made progress in understanding how certain changes in the DNA of soft
tissue cells cause sarcomas to develop. This information is already being applied to new tests
to diagnose and classify sarcomas. This is important because accurate classification helps
doctors select the most appropriate treatment. It is hoped that this information will soon lead
to new strategies for treating these cancers, based on specific differences between normal and
malignant soft tissue cells.
Classification
Classification of most cancers, including sarcomas, is based mostly on the way they look
under a microscope. Recent research has shown that several different kinds of soft tissue
sarcomas can look very similar under the microscope. By using new lab methods, researchers
discovered that most cancers that used to be called malignant fibrous histiocytoma (MFH)
are actually high-grade forms of liposarcoma, rhabdomyosarcoma, leiomyosarcoma, other
sarcomas, and even carcinomas or lymphomas. About 10% to15% of cancers called MFH
before, still cannot be given a precise classification, and these are now called pleomorphic
undifferentiated sarcomas or undifferentiated pleomorphic sarcomas (although the current
classification system of the World health Organization permits use of MFH as an alternate
name).
Chemotherapy
Active research in chemotherapy for soft tissue sarcomas includes studies of new drugs and
new ways to give drugs now available.
A new drug called trabectedin (Yondelis) has been shown to help some patients with soft
tissue sarcomas. It is approved for use in Europe, but it is still being tested in the United
States. In this country, it is currently only available as part of a clinical trial.
Targeted therapy
Even more active than research into chemotherapy is research into so-called targeted drugs.
These are drugs that specifically block molecules in the cancer cells that cause the cancers to
grow.
Other targeted drugs may also be helpful against sarcomas. For example, the targeted drug
sunitinib (Sutent) seems to slow the growth of many sarcomas. Sirolimus (Rapamune) has
shown some promise in treating patients with PEComa, especially pulmonary
lymphangioleiomyomatosis. Other drugs being studied include cixutumumab, PD0332991,
and ridaforolimus.
Anti-angiogenesis drugs
Drugs that block new blood vessel formation may help kill sarcomas by preventing their
nourishment by the blood vessels. One such drug, bevacizumab (Avastin) has shown a
small benefit in sarcoma patients, when given with doxorubicin (Adriamycin). Cediranib is a
newer anti-angiogenesis drug that shows promise in treating a certain kind of soft tissue
sarcoma.
Talking With Friends and Relatives About Your Cancer (also in Spanish)
Helping Children When a Family Member Has Cancer: Dealing With Diagnosis (also
available in Spanish)
Caring for the Patient With Cancer at Home: A Guide for Patients and Families (also
available in Spanish)
Guide to Controlling Cancer Pain (also available in Spanish)
Genetic Testing: What You Need to Know
When Cancer Doesnt Go Away
Hospice Care
Your American Cancer Society also has books that you might find helpful. Call us at 1-800227-2345 or visit our bookstore online at cancer.org/bookstore to find out about costs or to
place an order.
No matter who you are, we can help. Contact us anytime, day or night, for information and
support. Call us at 1-800-227-2345 or visit www.cancer.org.
Singer S, Maki R, OSullivan B. Soft tissue sarcoma In: DeVita VT, Heilman S, Rosenberg
SA, eds. Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia, Pa: Lippincott
Williams & Wilkins; 2011:1533-1577.
Chugh R, Wathen JK, Patel SR, et al. Efficacy of imatinib in aggressive fibromatosis: Results
of a phase II multicenter Sarcoma Alliance for Research through Collaboration (SARC) trial.
Clin Cancer Res. 2010 Oct 1;16(19):48844891.
Clark MA, Fisher C, Judson I, et al. Soft-tissue sarcomas in adults. New Engl J Med.
2005;353:701711.
Cormier JN, Pollock RE. Soft tissue sarcomas. CA Cancer J Clin. 2004;54:94109.
Dei Tos AP. Classification of pleomorphic sarcomas: where are we now? Histopathology.
2006 ;48:5162.
Demetri GD, Chawla SP, Ray-Coquard I, et al. Results of an international randomized phase
III trial of the mammalian target of rapamycin inhibitor ridaforolimus versus placebo to
control metastatic sarcomas in patients after benefit from prior chemotherapy. J Clin Oncol.
2013 Jul 1;31(19):2485-92. Epub 2013 May 28.
Dickson MA, Tap WD, Keohan ML, et al. Phase II trial of the CDK4 inhibitor PD0332991 in
patients with advanced CDK4-amplified well-differentiated or dedifferentiated liposarcoma.
J Clin Oncol. 2013 Jun1;31(16):2024-20248. Epub 2013 Apr 8.
George S, Merriam P, Maki RG, et al. Multicenter phase II trial of sunitinib in the treatment
of nongastrointestinal stromal tumor sarcomas. J Clin Oncol. 2009 Jul 1;27(19):31543160.
Epub 2009 May 18.
Kummar S, Allen D, Monks A, et al. Cediranib for metastatic alveolar soft part sarcoma. J
Clin Oncol. 2013 Jun 20;31(18):2296-2302. Epub 2013 Apr 29.
Le Cesne A, Domont J, Cioffi A, et al. Mapping the literature: role of trabectedin as a new
chemotherapy option in advanced pretreated soft tissue sarcoma. Drugs Today (Barc).
2009;45:403421.
Maki RG, D'Adamo DR, Keohan ML, et al. Phase II study of sorafenib in patients with
metastatic or recurrent sarcomas. J Clin Oncol. 2009 Jul 1;27:31333140.
Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F. PEComas: the past, the present
and the future. Virchows Arch. 2008 Feb;452(2):119132. Epub 2007 Dec 14.
McCormack FX, Inoue Y, Moss J, et al. Efficacy and safety of sirolimus in
lymphangioleiomyomatosis. N Engl J Med. 2011 Apr 28;364(17):15951606. Epub 2011
Mar 16.
Nascimento AF, Raut CP. Diagnosis and management of pleomorphic sarcomas (so-called
"MFH") in adults. J Surg Oncol. 2008;97:330339.